(1139) A Rare Case of Acute Presentation of Granulomatosis With Polyangiitis

Introduction: ANCA-associated vasculitis is a category of diseases that predominantly affect small-medium vessels. Granulomatosis with polyangiitis (GPA) is a rare condition that causes necrotizing granulomas affecting the upper respiratory tract, lungs and kidneys. We present a rare case of undiagnosed GPA in a patient who required plasmapheresis due to life threatening organ failure.

Description: 73-year-old female with a past medical history of hypertension and heart failure presented to the hospital with a one-day history of shortness of breath and cough. She was found to be hypoxic at 70% and placed on 5 liters of oxygen. Physical examination was unremarkable and bloodwork revealed a creatinine of 4.2 and hemoglobin of 6.9. Hb stabilized after one unit of pRBC. Chest CT concerning for pulmonary edema. She acutely decompensated and required intubation, during which copious amounts of blood was noted in her oral cavity and nasogastric tube. A bronchoscopy with BAL was performed and sequential alliquots concerning for DAH. Given her acute renal failure and bronchoscopy findings, this was concerning for ANCA-vasculitis and she was started on high dose steroids and continuous renal therapy. Further bloodwork showed positive ANA, p-ANCA and anti-MPO antibodies, consistent with a diagnosis of GPA. The patient improved after receiving two doses of Rituximab and seven plasmapheresis treatments.

Discussion: GPA is a rare autoimmune condition of the small-medium blood vessels. It has an incidence of 0.4-11.9 cases per million persons per year and affects males more than females. Lung involvement can cause diffuse pulmonary hemorrhage, which significantly contributes to morbidity and mortality. Renal involvement is seen in majority of cases, which can lead to CKD or ESRD. Treatment includes a course of Rituximab and glucocorticoids, but life-threatening symptoms may require plasmapheresis. GPA carries an increased morbidity and mortality prognosis due to either irreversible organ dysfunction or complications of immunosuppressive therapy. Due to the critical presentation of our patient, it was deemed that she was a candidate for plasmapheresis. It is important to recognize the variety of presentations that GPA can have, as early recognition and treatment can be life-saving with a 5-year survival rate of 74%.